【摘要】：先天性肺表面物質(zhì)功能障礙疾病(surfactant dysfunc-tion disorders)又稱先天性肺表面物質(zhì)代謝缺陷(Inborn er-rors of surfactant metabolism,IESM),主要指SP-B基因(SFTPB)、SP-C基因(SFTPC)、ABCA3基因(ABCA3)、甲狀腺轉(zhuǎn)錄因子-1基因(NKX2.1)和粒細(xì)胞-巨噬細(xì)胞集落刺激因子受體基因(GMCSFR)的突變或缺失導(dǎo)致的一類疾病。這類疾病目前被認(rèn)為是兒童彌漫性間質(zhì)性肺疾病的重要原因。其發(fā)病年齡和嚴(yán)重程度變化很大,從引起足月新生兒致死性呼吸窘迫綜合征,到嬰兒期、兒童期和成年人的彌漫性肺間質(zhì)疾病,甚至到成年人出現(xiàn)肺纖維化。
[Abstract]:Congenital pulmonary surface substance dysfunction (surfactant dysfunc-tion disorders), also known as congenital pulmonary surface substance metabolic deficiency (Inborn er-rors of surfactant metabolism,IESM), mainly refers to a class of diseases caused by the mutation or deletion of SP-B gene (SFTPB), SP-C gene (SFTPC), ABCA3 gene (ABCA3), thyroid transcription factor-1 gene (NKX2.1) and granulocyte-macrophage colony-stimulating factor receptor gene (GMCSFR). This kind of disease is now considered to be an important cause of disseminated interstitial lung disease in children. The age and severity of onset vary greatly, from fatal respiratory distress syndrome in full-term neonates to disseminated pulmonary interstitial diseases in infancy, childhood and adults, and even pulmonary fibrosis in adults.
【作者單位】： 深圳市兒童醫(yī)院呼吸科;
【分類號(hào)】：R563.9

【相似文獻(xiàn)】

相關(guān)期刊論文 前3條

1 ;氣是物質(zhì)功能和理三者的合一[J];山東中醫(yī)雜志;1992年05期

2 章增加;論中醫(yī)氣的物質(zhì)功能雙重屬性及其意義[J];廣西中醫(yī)藥;1994年03期

3 王敏;牛力;;大美不言中國(guó)茶[J];東方養(yǎng)生;2009年07期

，

本文編號(hào)：2525985